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[Surgical treatment of multiple epiphyseal dysplasia in the hip joints in childhood--short-term results].

PURPOSE OF THE STUDY: The presentation of our results of surgical treatment of the hip joints affected by multiple epiphyseal dysplasia, using the Steel technique of triple osteotomy of the innominate bone in children. MATERIAL: Twelve hip joints in 11 patients were treated by the Steel technique of triple osteotomy of the innominate bone at our department in the period from 2000 to 2001. However, the evaluation involved only children who also had multiple epiphyseal dysplasia, i.e., two boys and one girl. One of the boys had bilateral surgery with 6.5 months between the operations. The average age at the time of operation was 7.5 years, with a range of 6.5 to 8.5 years. The indication for the Steel osteotomy included severe conditions characterized by flattening and fragmentation of the proximal femoral epiphysis that was insufficiently covered by a dysplastic acetabulum. METHODS: The clinical evaluation was based on a classification system according to Merle d'Aubigné and Postel. Assessment was made on X-ray films before and after surgery. The follow-up was 17 to 29 months with an average of 2 years. RESULTS: The average Merle d'Aubigné and Postel score for the operated-on hips was 17.5 points. The maximum attainable value is 18 points; therefore all hips showed excellent clinical outcomes. Radiological examination showed an improvement in Wiberg's center-edge (CE) angle, i.e., from 15 degrees preoperatively to 44 degrees postoperatively (range, 10 to 25 and 41 to 48 degrees, respectively). The surgical treatment resulted in an average increase in the CE angle by 29 degrees, with the range between 21 and 33 degrees according to correction extent. DISCUSSION: A comparison of our results with those of other authors was not possible since no literature data were available on the Steel osteotomy in patients with multiple epiphyseal dysplasia. In our opinion these children constitute a specific category of patients with this disorder but the size of our group was too small to draw conclusions with a more general validity. CONCLUSIONS: The Steel triple osteotomy of the innominate bone permits correction of abnormal anatomical conditions of hip joints in severe multiple epiphyseal dysplasia. The nature of this disorder, however, requires a long-term follow-up of the patients treated.

Child↗

[Effects of an epiphyseal peptide preparation epithalamin on serotonin metabolism in the pineal gland of rats].

5-day morning injections to pubertal male rats of polypeptide epithalamin preparation obtained from cattle epiphysis, in dose of 0.25 mg/100 g body mass induced the increase of serotonin epiphyseal concentration night peak, N-acetylserotonin and melatonin and didn't produce any essential influence on 5-methoxytryptamine, 5-oxy- and 5-methoxyindoleacetic acid level. It has been concluded, that epiphyseal peptides and indoles interact according to ultrashort connection, epiphyseal peptides point of application is the reaction of tryptophan transformation into serotonin and its further metabolism in N-acetylserotonin and melatonin. It has been suggested that the increase of epiphyseal melatonin production is on the basis of epithalamin therapeutic action.

5-Methoxytryptamine↗

Epiphyseal chondroblastoma (a study of 39 cases).

A fully documented series of thirty nine cases of epiphyseal chondroblastoma is described. This is a remarkable series because of the rarity of this tumour. There is a slight predilection for the male sex. The age most affected is ten to twenty years. The tumour progresses slowly and joint involvement and pain are slight. The commonest site is the proximal epiphysis of the humerus, followed by the epiphyses of the knee. The classical appearances are of a clearly defined area of osteolysis, central or eccentric, with foci of calcification, in the epiphyseal or apophyseal regions, and often transgressing the epiphyseal cartilage. We have never observed involvement of the opposite bone in the affected joint. In four of our thirty nine cases the neoplasm invaded the point and/or soft tissues. The differential diagnosis, especially in localisations at the knee, is with giant cell tumour. The tumour is slow growing and the prognosis is always good. We have never seen malignant transformations or so-called "benign" pulmonary metastases. The few recurrences in this series (five out of thirty nine) were all cured by a second operation. Curettage and grafting is the operation of choice.

Adolescent↗

[Disorders of vascularization in para-epiphyseal groove humeral fractures and their clinical significance for the growing skeleton].

We analysed retrospectively 50 humerus fractures in the vicinity of elbow joint and 40 fractures of the proximal humerus with respect to possible injuries of the vessel pattern of the epiphyseal plate. The normal vessel pattern of the epiphyseal plate was examined postmortem on 8 foetus and newborns up to 6 months. We plastinated the affected regions and could compare symmetry of the vessel pattern in the epiphyseal plates as well as therapeutic consequences, especially after injuries of the elbow joint. Vascular disturbances in the case of young children, i.e. by fractures of the lateral oder medial condyle are undoubtedly seen. A fish-tail deformity can be a complication. By older children with mature epiphyseal plate we could not register any complications. In such cases we are interested in restoring the joint stability by refixation.

Arteries↗

[A special form of dominant spondylo-epiphyseal dysplasia (author's transl)]].

A special form of dominant spondylo-epiphyseal dysplasia has been observed in a 3 year-old boy and in his father. The roentgenological examination showed alterations involving chiefly the upper femoral extremities and the spine. This might suggest the diagnosis of spondylo-epiphyseal dysplasia congenita. However the clinical features were different. The alterations of the growth plate were also different from those found in typical forms of spondylo-epiphyseal dysplasia congenita. Large dilatations of the rough endoplasmic reticulum of chondrocytes are present in both syndromes but their content had different histochemical features. The cartilage alterations were also different from those found in the dominant form of poly-epiphyseal dysplasia as well as in pseudo-achondroplasia.

Adult↗

Distribution and subcellular immunolocalization of 1,25-dihydroxyvitamin D3 receptors in rat epiphyseal cartilage.

The distribution and subcellular localization of the 1,25-dihydroxyvitamin D3 receptor (VDR) in the epiphyseal cartilage of normal weaning rats were examined immunocytochemically at the light and electron microscope level using a monoclonal anti-VDR antibody (9A7 gamma). VDR immunoreactivity was detected in the nuclei of chondrocytes in all zones of the epiphyseal plate cartilage from the resting to calcifying chondrocytes, and at much lower concentrations, in the cytoplasms. Perichondrial mesenchymal cells contained no VDR immunoreactivity. VDR immunoreactivity developed in the nuclei of cells in the lateral margin area as they acquired the chondroblast phenotype. VDR immunoreactivity was also found over the nucleoli of chondrocytes in all cells zones of the epiphyseal plate and appeared in the nucleoli of the cells in the lateral margin area before immunostaining of the nuclei, as the mesenchymal cells differentiated into chondroblasts. Electron microscopy showed that the immunoreactivity for 1,25(OH)2D3 receptor, indicated by gold particles, was associated with scattered clumps of compact chromatin and small clumps of dispersed chromatin. But the nuclei immunostaining patterns before and after mitosis were different in proliferative chondrocytes. The heterochromatin along the nuclear envelope was immunonegative in interphase chondrocytes, but there was VDR immunostaining over the rim of the perinuclear chromatin just after mitosis. In the nucleoli, the dense fibrillar component was immunostained, but the fibrillar centers and the perinuclear chromatin were not. This distribution of VDR immunoreactivity suggests that the hormone is directly involved in differentiation, proliferation and maturation of cartilage cells, and also with extracellular calcification in epiphyseal cartilage. The presence of immunoreactive VDR receptors in nucleoli of chondrocytes, particularly the fibrillar component, suggests that 1,25(OH)2D3 may be involved in regulation of ribosomal genes.

Animals↗

Shear strength of the human femoral capital epiphyseal plate.

After the determination of the shear strength and modes of failure of the capital femoral epiphyseal plates in twenty-five pairs of hips obtained post mortem from children five days to fifteen years and ten months old, with the plate intact on one side and with the perichondrial fibrocartilaginous complex removed on the other, the gross and microscopic morphology was studied. The shear strength of the human epiphyseal plate varied with age and was greatly dependent on the surrounding perichondrial complex in infancy and early childhood, but less so in adolescence. When this complex was excised, the strength of the epiphyseal plate was diminished, especially in specimens from younger children. The forces necessary to cause slipping were found to be within the physiological range of the force that would be generated in overweight children, suggesting that purely mechanical factors may play a major role in the etiology of slipped capital femoral epiphysis.

Adolescent↗

[Epiphyseal fractures-dislocations of the lower extremity of the tibia].

Ninety six distal tibial epiphyseal fractures were identified and treated in our institution from 1976 to 1988. The average age was twelve years and eight months (range two to seventeen years), but seventy-one were between eleven and fourteen years old. Using the Salter-Harris classification we have found twelve type 1 tibial fractures, fourty-two type 2, thirty type 3 and twelve type 4. Four were triplane fractures and seven were Tillaux fractures. Twenty-six had injuries in the medial corner of the ankle mortise (Mac-Farland). Fifty patients were treated non-operatively with closed reduction and plaster cast. Fourty-six fractures were treated surgically. Seventy patients were available for follow-up evaluation. The average follow-up was thirty-two months (range 6 months to eleven years). The tibial distal epiphyseal cartilage was closed in 48 patients. As short-term complications we have seen three post-operative displacements after closed reduction; all of them were treated surgically. Five incomplete closed reduction needed open reduction needed open reduction and bone fixation. Two infections occurred after a surgical approach. Among late complications we have seen eleven premature epiphyseal cartilage closure (rate 15%). Four were responsible of angular deformities. One child has a tibial osteotomy for varus deformity after a medial closure. Two ankle arthritis occurred: one of them was seen after a post-operative infection. In two cases of fracture of the medial mortise corner, a valgus deformity with hypertrophy of the medial malleolus occurred. Ankle arthritis is the most severe complication of the adolescent articular fractures (Tillaux and triplane fractures).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Necrosis of the proximal epiphyseal plate cartilage of the femur following detachment of the perichondral ring. Its relationship to the etiology of proximal epiphysiolysis of the femur.

A histological study of the proximal epiphyseal plate cartilage of rabbit femurs following detachment of the perichondral ring was performed. 15 animals were used, 5 of which being controls. Slight to severe necrotic changes in the plate were present in 9 out of 10 animals. In severe necrosis there was formation of fissures similar to those seen in human femoral epiphysiolysis. The necrosis was considered ischemic in nature and due to the detachment of the perichondral ring with involvement of the blood supply for the femoral epiphysis. Also the epiphyseal and metaphyseal bone marrow and bone tissue, as well as the articular cartilage, presented necrotic changes in a few animals. Such necrosis of the epiphyseal plate may be a factor in the pathogenesis of human proximal epiphysiolysis of the femur.

Animals↗

Angel-shaped phalango-epiphyseal dysplasia (ASPED): identification of a new genetic bone marker.

We describe a "new" mild malformation of the phalanx, which we call the "angel-shaped phalanx" (ASP) because of its resemblance to the little angels used for the decoration of Christmas trees. A particular middle phalangeal type of ASPs is found in a distinct variety of multiple epiphyseal dysplasia with marked retardation of bone age and severe coxarthrosis in adult life, previously reported as "hereditary peripheral dysostosis" [Bachman, 1967: Proc R Soc Med 60:21-22; Giedion, 1969: Fortschr Rontgenstr 110:507-524]. However, these authors overlooked the unique configuration of the middle phalanges. We renamed the condition "angel-shaped phalango-epiphyseal dysplasia (ASPED)", which may be transmitted in an autosomal-dominant manner. Six new patients are added, bringing the total to nine patients (two families and two isolated patients). ASPs were seen in five of six children. The ASPs grew into inconspicuous brachydactyly after physeal closure (3/3). The most important additional radiological finding is late and dysplastic development of both femoral heads (5/5), leading to Perthes-like and osteoarthritic changes and severe hip pain in the early thirties (2/2 adults, having reached this age). The marked retardation of carpal bone age may lead to unnecessary clinical evaluation for endocrine disorders. Less frequent clinical manifestations of ASPED are hyperextensibility of the interphalangeal joints (7/9) and hypodontia (4/7). Other types of ASPs are observed in brachyphalangy type C, spondylo-megepiphyseal-metaphyseal dysplasia, and other conditions. The concept of mild bone abnormalities as specific markers for genetic disease, as with cone-shaped epiphyses and now evident in ASPED, may also be useful for ASPs in general.

Adolescent↗

Glucose 6-phosphatase and glycogen phosphorylase activities in chondrocytes in epiphyseal cartilage of growing rats.

Glycogen, glycogen phosphorylase, and glucose 6-phosphatase (G6Pase) activities were examined cytochemically in chondrocytes of femoral epiphyseal cartilages and cartilaginous ribs of 3- and 7-day-old rats. G6Pase activity was also examined biochemically. Glycogen was abundant in chondrocytes of the reserve zone, while it became scarce in the cells of the proliferative zone. From the upper part (adjoining the proliferative zone) to the lower part of the hypertrophic zone, glycogen accumulated in chondrocytes and decreased in the cells of the degenerative zone. Inversely, glycogen phosphorylase a and G6Pase activities were relatively high in chondrocytes of the proliferative zone and upper hypertrophic zone and were low in the cells of the reserve zone, lower hypertrophic zone, and degenerative zone. The reaction product for G6Pase was present in the endoplasmic reticulum and nuclear envelope of all types of chondrocytes composing the cartilages, although the amounts of reaction product varied with the cell types in parallel with the histochemical results. Biochemical G6Pase activity was higher in epiphyseal cartilages than in cartilaginous ribs. The possible mechanism and significance of the accumulation and decrease of glycogen in chondrocytes of the epiphyseal cartilage were discussed.

Animals↗

Sonographic identification and measurement of the epiphyseal ossification centers as markers of fetal gestational age.

PURPOSE: This study was conducted to verify the predictive value of epiphyseal ossification center measurements in estimating gestational age. METHODS: Women with singleton pregnancies of 30-40 weeks gestation (n = 377) were enrolled in this prospective study. The distal femoral, proximal tibial, and proximal humeral ossification centers were identified and measured. A nomogram of fetal bone development was created using the sum of the three diameters. RESULTS: Gestational age correlated well with the diameters of the distal femoral and the proximal tibial epiphyseal ossification centers but even better with the sum of the three ossification centers. Positive predictive values of the fetus having gestational age of at least 37 weeks when the sum of the three centers was 7, 11, and 13 mm were 82%, 94%, and 100%, respectively. A nomogram was created using the sum of the ossification centers for 30-40 weeks' gestational age. CONCLUSIONS: Ultrasonographic visualization of the epiphyses ossification centers may be a useful marker of fetal gestational age.

Adolescent↗

Tibial epiphyseal development: a cross-sectional histologic and histomorphometric study in the New Zealand white rabbit.

Sequential histomorphometric studies on the developing rabbit tibia from birth to skeletal maturity demonstrate that growth plate height lessens as longitudinal growth diminishes. Differing rates of development proximally and distally are documented. Distally, growth plate height, width, and area and total epiphyseal area peak or reach near maximum values by 3 weeks, whereas proximally, they do so by 8 weeks (except for height, which also peaks at 3 weeks). The distal growth plate is being obliterated by 16 weeks, at which time the proximal growth plate remains well structured and open. The distal tibia and fibula develop as one tissue mass. The articular cartilage and epiphyseal cartilage are continuous from birth, whereas a single ossification center and a single growth plate are present by 8 weeks. The data point to the presence of intrinsic growth plate, as well as systemic, control mechanisms affecting skeletal growth. Knowledge of temporal and quantitative features of epiphyseal and growth plate development will greatly aid in the elucidation of the underlying controls.

Aging↗

Limb lengthening by low rate epiphyseal distraction. An experimental study in the caprine tibia.

Low-rate epiphyseal distraction of the left proximal tibia using a modified bilateral Hoffmann external fixation device, was accomplished in 14 goats (age 6-7 months). Daily distraction at a rate of 0.5 mm was continued for 5.5 weeks. Epiphysiolysis occurred after 5-9 days. After distraction had been discontinued, the external device was left in situ for fixation for 4 weeks. The animals in group 1 (n = 6), group 2 (n = 3), and group 3 (n = 5) were killed at 4, 16, and 32 weeks, respectively, from the end of the distraction period. The proximal growth plate of the elongated tibia appeared radiolucent at the conclusion of the study but was less distinct and reduced in height as compared with the control leg. The gained length at the end of the distraction period was on the average 2.2 cm (11.9%). This gain in length was reduced with further growth. In group 3, the final gain in length was reduced to half. A reduced cortical thickness of the bone within the area subjected to distractional force was observed. This finding is attributable to stress protection. An interesting observation comparing control and operated tibia was an increase of the cross-sectional diameter of the elongated bone. Both tibiae of all animals were tested mechanically. The torsional strength of the elongated tibiae as compared with controls was on the average 29% in group 1, 79% in group 2, and 95% in group 3. We conclude that leg lengthening by low-rate epiphyseal distraction in the proximal tibia is a justifiable alternative to other lengthening procedures in individuals approaching adolescence. In juveniles, lengthening by epiphyseal distraction may result in reduction in the longitudinal growth.

Animals↗

Distribution of elastic fiber types in the epiphyseal region.

Three types of elastic fibers have been described: mature elastic fibers, elaunin fibers, and oxytalan fibers. To our knowledge, their location in the immature epiphysis has never been previously reported. The aim of the present study was to use histochemistry, immunohistochemistry, and electron microscopy to demonstrate the distribution of each type of elastic fiber in the epiphyseal region of growing humans and rabbits. Histological samples were collected from the knees of 10 immature New Zealand White rabbits and four children of various ages. The Weigert resorcin-fuchsin, Gomori aldehyde-fuchsin, Verhoeff iron haematoxylin, and Fullmer-Lillie methods were used for histochemistry; anti-elastin monoclonal antibodies, for immunohistochemistry; and tannic acid, uranyl acetate, and lead citrate stain, for transmission electron microscopy analysis. Elastic fibers were detected in the perichondrium, the epiphyseal vessels, and the outer and middle zones of Ranvier's groove. Their orientation was longitudinal in the outer zone and circumferential in the middle zone of the groove. Oxytalan fibrils (i.e., bundles of filaments of 10-12 nm in diameter that do not contain elastin) and elaunin fibers (i.e., filaments that cross discontinuous aggregates of elastin) were more plentiful in the middle zone and decreased with age, whereas mature fibers were more numerous in the outer zone and increased with age. This organization of elastic fibers seems to indicate an age-related process of maturation of the elastic network. The contribution of these fibers to the mechanical properties of the epiphyseal plate and to the growth process remains to be determined.

Age Factors↗

The ultrastructure of osteochondrosis of the articular-epiphyseal cartilage complex in growing swine.

Osteochondrosis of the articular-epiphyseal cartilage complex (A-E complex) is a significant clinical disease in swine. It has been suggested that osteochondrosis is the underlying cause of osteochondritis dissecans in humans. The purpose of this investigation was to characterize the ultrastructural changes in the earliest macroscopically visible lesion of the epiphyseal cartilage in osteochondrosis of the A-E complex in swine. Osteochondritic epiphyseal cartilage from the distal femora and humeri of growing crossbred boars was collected, embedded in plastic, and studied light and electron microscopically. The predominant lesion was chondronecrosis, characterized by chondrocyte death and loss of matrical proteoglycan. Transition from normal to abnormal cartilage was abrupt. Lipid accumulated in chondrocytes within and adjacent to lesions, but not in chondrocytes distant from lesions. Intracellular lipid accumulation was an important feature of the lesion and may play a role in its initiation. It is hypothesized that intracellular lipid accumulation results from hypoxia/anoxia and may precede matrix degeneration, which precedes cell death.

Animals↗

[Correction of the leg axis after epiphyseal fracture and progressive abnormal growth of the proximal tibia].

Development of an angular deformity around the knee joint, following a posttraumatic premature epiphyseal closure is a rare but serious complication. We present a case report of this complication following a proximal tibial epiphyseal injury in a 9 year old child initially treated conservatively with plaster immobilization. Subsequently, partial closure of epiphysis on medial side resulted in genu varum of 20 degrees, which was treated with medial open wedge osteotomy of the proximal tibia combined with resection of a segment from the proximal fibula, and a percutaneous epiphysiodesis of the proximal tibia and fibula. At three years follow up, the child had shortening of the leg by 1 cm, but no angular deformity. Significance of regular follow-up after an epiphyseal injury to detect the condition and role of operative management with various modalities is discussed.

Casts, Surgical↗